Bilateral Posterior and Lateral Semicircular Canals Aplasia Associated with Other Inner Ear Malformation in A 2-Year-Old Child: A Rare Case Report
Journal Title: Scholarly Journal of Otolaryngology - Year 2018, Vol 1, Issue 4
Abstract
Inner ear malformation accounts for approximately 20% of reported cases of congenital sensorineural deafness. It results from developmental arrest in different stages of embryogenesis. Aplasia of one of the semicircular canals with or without cochlear malformation is well known and has been reported in journals. However; to our knowledge, aplasia of two semicircular canals have been rarely reported, and for this reason the clinical reflection of this anomaly is poorly understood. This case will be hopefully used to gain an in-depth understanding of illness and help in the diagnosis and proper management of future cases. In this paper we present a report concerns a 2-year-old boy with no family history of hearing loss, presenting with bilateral profound sensorineural hearing loss with no other congenital anomalies. CT scan and MRI shows dysplastic bilateral vestibules, bilateral hypoplastic cochlea and bilateral lateral and posterior semicircular canal (SCC) aplasia. Inner ear in embryo starts to develop during 3rd week of gestation. A structure called ‘otic placodes’ which arise from the surface ectoderm on each side of the rhombencephalon represent the fundamental embryological part of inner air developmental. The otic placodes subsequently invaginate and form otocysts. Diverticulum buds from the otocysts form the endolymphatic sacs, which eventually by the 5th week; form the cochlea and vestibules, while utricle segments of the otocysts form the semicircular canals by the end of the 7th week. The Superior semicircular canal develops first while the lateral develops last [1]. Premature arrest in different parts of development leads to inner ear malformation. The earlier the arrest the more sever the deformity [2]
Authors and Affiliations
Fatimah Albazroun, Zahrah Almoumen, Hazim Al Eid
Keywords
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- EP ID EP599122
- DOI 10.32474/SJO.2018.01.000116
- Views 36
- Downloads 0
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